Grażyna Sobol-Milejska

The Immunophenotypes of Blast Cells in B-Cell Precursor Acute Lymphoblastic Leukemia: How Different Are They from Their Normal Counterparts?

Currently, there are three major maturational stages of CD19 antigen expressing B‐cell precursors (hematogones). In B‐cell precursor acute lymphoblastic leukemia (BCP‐ALL), the malignant counterpart of hematogones, the leukemic blasts share common phenotypic features. The aim of the study was to enumerate the actual differences between the leukemic blasts in the CD10+ and CD10− subgroups of BCP‐ALL and hematogones by assessing the expression of the antigens: TdT, CD34, CD45, CD10, CD38, CD20 and CD22.

Inflammatory Myofibroblastic Tumor of the Heart in the Infant: Review of the Literature.

Primary heart tumors are extremely rare, constituting approximately 0.02% of all malignancies. Inflammatory myofibroblastic tumor (IMT) constitutes <5% of primary heart tumors. Until now, IMT of the heart has been described in 21 infants below 1 year of age. Its etiology remains unknown. IMT usually develops within the right atrial and ventricular endocardium. The main clinical symptoms reported in the affected infants involved increasing respiratory failure, cyanosis, and heart murmurs. Histopathologically, IMT is characterized by the myofibroblast proliferation with inflammatory infiltrates composed of plasmocytes, lymphocytes, and histiocytes. Tumor resection is the treatment of choice in IMT. Such tumor location is associated with the high risk of perioperative failure. Steroid therapy and chemotherapy is reported in the literature as a nonsurgical treatment alternative. Here, we present a review of clinical symptoms, diagnostic and treatment options, based on published case reports of IMT in infants, including our 11-month-old patient with IMT located within the pericardium.

Outcome of acute lymphoblastic leukemia in children with down syndrome—Polish pediatric leukemia and lymphoma study group report

ABSTRACT Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group—14 patients, an intermediate-risk group—24, a high-risk group—3. All patients were treated according to ALLIC 2002 protocol. The median observation time of all patients was 6.1 years, and in patients with DS 5.3 years. Five-year overall survival (OS) was the same in all patients (86% vs 86%, long-rank test, p = .9). The relapse-free survival (RFS) was calculated as 73% in patients with DS and 81% in patients without DS during a median observation time (long-rank test, p = .3). No statistically significant differences were found in the incidence of nonrelapse mortality between those two groups of patients (p = .72). The study was based on children with ALL and Down syndrome who were treated with an identical therapy schedule as ALL patients without DS, according to risk group. This fact can increase the value of the presented results.

Serum levels of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in children with brain tumours

BACKGROUND Angiogenesis is the process of new vessel formation originating from the existing vascular network. It plays an important role in the growth and spread of malignancies, including brain tumors. The process of angiogenesis is characterized by increased expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and by the release of their soluble forms into circulation. OBJECTIVES The aim of the study was to evaluate serum levels of VEGF and bFGF in children with malignant and benign brain tumors. MATERIAL AND METHODS The study group (group N) included 106 children diagnosed with brain tumors. The children in group N were classified according to tumor pathology into 3 subgroups: N1 (n = 63): patients with malignant tumors, excluding anaplastic astrocytoma (AA) and glioblastoma multiforme (GBM); N2 (n = 25): patients with benign tumors; and N3 (n = 18): patients with high grade gliomas (AA and GBM). VEGF and bFGF were determined by ELISA in blood samples before the initiation of chemotherapy. VEGF and bFGF levels were compared within the subgroups in relation to tumor grading and the extent of surgery. RESULTS The median VEGF in patients with brain tumors was significantly higher than in the control group. The median levels of VEGF and bFGF in subgroup N1 were significantly higher than in the control group. The differences in VEGF and bFGF concentrations between the subgroups in relation to the extent of tumor resection were not significant. CONCLUSIONS Significantly higher plasma VEGF levels in children with brain neoplasms may reflect enhanced angiogenesis in the tumors.

Surface expression of Cytokine Receptor-Like Factor 2 increases risk of relapse in pediatric acute lymphoblastic leukemia patients harboring IKZF1 deletions

We prospectively examined whether surface expression of Cytokine Receptor-Like Factor 2 (CRLF2) on leukemic blasts is associated with survival and induction treatment response in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) patients. Flow cytometric analysis of bone marrow-derived leukemia cells revealed that 7.51% (29/286) of 386 pediatric BCP-ALL patients were CRLF2-positive (CRLF2pos) at diagnosis. The median minimal residual disease (MRD) was lower in CRLF2pos than CRLF2-negative (CRLF2neg) patients on day 15 (MRD15) after induction therapy [0.01% (0.001-0.42%) vs. 0.45% (0.05-3.50%); p=0.001]. By contrast, the MRD15 was higher in Ikaros family Zinc Finger Protein 1 (IKZF1)-deleted BCP-ALL patients than in BCP-ALL patients without IKZF1 deletions [1.18% (0.06-12.0%) vs 0.33% (0.03-2.6%); p=0.003]. Subgroup analysis showed that MRD15 levels were lower in IKZF1Δ/CRLF2pos patients than in IKZF1Δ/CRLF2neg patients [0.1% (0.02-5.06%) vs. 2.9% (0.25-12%); p=0.005]. Furthermore, MRD15 levels were higher in IKZF1WT/CRLF2neg patients than in IKZF1WT/CRLF2pos patients [0.40% (0.04-2.7%) vs. 0.001% (0.001-0.01%)]. Despite the low MRD15 levels, IKZF1Δ/CRLF2pos patients showed poorer relapse-free survival (RFS) than other patient groups (p=0.003). These findings demonstrate that surface CRLF2 expression is associated with increased risk of relapse in pediatric BCP-ALL patients harboring IKZF1 deletions.

Blastic Plasmocytoid Dendritic Cell Neoplasm With Skin and Myeloid Location.

Blastic plasmocytoid dendritic cell neoplasm is one of the aggressive hematopoietic malignancy with predilection for the skin. It is a tumor derived from the plasmocytoid dendritic precursor cells. Skin is its most common location; however, it may also affect bone marrow, lymph nodes, or spleen. The condition is rarely diagnosed in children; so far only 36 cases have been reported. The diagnosis usually takes time and there are no uniform treatment guidelines. This paper presents a case of a 6-year-old boy with blastic plasmocytoid dendritic cell neoplasm (skin and myeloid location). He has been treated according to the acute lymphoblastic leukemia treatment protocol.

Assessment of angiogenesis in children with acute lymphoblastic leukemia based on serum vascular endothelial growth factor assay

Introduction: Vascular endothelial growth factor A (VEGFA) is a key proangiogenic cytokine. The role of angiogenesis in acute lymphoblastic leukemia (ALL) is still unclear. The purpose of the study was to assess angiogenesis in children with ALL based on serum VEGFA level determined at diagnosis and at remission with further participant subdivision into different risk groups. Materials and Methods: Forty children, aged 3–12 years (mean age: 8 years) with newly diagnosed ALL, were enrolled in the study. The control group (Group C) was twenty healthy children. According to the risk assessment, they were classified into a standard-risk group, an intermediate-risk group (IRG), or a high-risk group (HRG). Results: The median serum VEGFA levels at diagnosis were significantly higher in IRG and HRG as compared to Group C. The VEGFA levels at remission were significantly higher in all study groups, as compared to Group C. The differences in median values of serum VEGFA levels between the study groups both at diagnosis and at remission were not statistically significant. Conclusions: The angiogenesis in ALL seems to be intensified at diagnosis as a result of neoplasmatic bone marrow rebuilding and at remission as its intensive recovering.